Thursday, June 9, 2011

Just another day in paradise......

By the end of the day I was wondering why things ALWAYS have to be so hard, and I was giving myself the weekly pep talk that our situation could be worse…

Starting any day off with only three hours of sleep is never good. If there is a woman out there who knows how to do a complete brain shut-down, I need to talk to you. Immediately. I attempted to go to bed early, but Gavin wasn't ready, as usual, and we ended up falling asleep around 10:45 pm. I guess my brain didn't get enough worrying in before I fell asleep because I woke up at 1:30 and wasn't able to go back to sleep until 3:30. And then I was back up at 4:00. Awesome. So, that is how my day began. Jacob, Brie, Gavin and myself were on the road by 5:15 and at Shands MRI on time at 7:30 am. Tired but ready.

I could really go off on how VERY upset I was when they told me Jacob had been taken off the schedule for his MRI and his place had been filled. You would think after eleven years spent in and out of the hospitals, clinics and doctors appointments that I would be a little more understanding to the mistakes that seem to ALWAYS happen to us. Well, I am not. Instead, I have no tolerance, nor patience, for all the "human errors." I'm sick of it. And the way that the entire ordeal was handled by the “professionals” only made my exhausted and nervous emotional state even more fragile. Jacob's orders and appointment papers were in my hands and they still spent over an hour trying to figure out what happened and "trying" to get Jacob in for his scan. Not once did they tell me they would take care of it, that they were sorry for the confusion or that Jacob would have his MRI. Totally unacceptable. I called the CT scan department to confirm Jacob's appointment with them during the MRI mix-up and I was informed that Jacob had been removed from there as well. YEAY! After another call to Jacob's nurse at Dr. Smith's office, his CT scan appointment was back in place. Again, I was incredibility disappointed with how we were treated when Jacob had completed the first part of his MRI. They came and got us from the lobby, informed us that Jacob was being sent to CT to get his IV and CT scan there and that he would come back to MRI for the imaging with contrast, the second half. Their reason being that another case needed the MRI bed Jacob was on. Really? At this point Jacob was starving and grumpy and I felt like steam could explode from my ears and eyes at any moment. Jacob wasn't allowed to eat two hours prior to CT so it was good that he was getting it over with, but the reasoning was wrong. So, at 9:50 we were shuffled to the basement CT scan department and put in another waiting room. I put more Lidocaine cream on Jacob's arm since MRI hadn't put his IV in yet (his port can handle MRI contrast, but not CT contrast). The tech came and got Jacob around 10:10, popped his IV right in (Jacob didn't feel a thing, which might have been the highlight of the day) and took his pictures. The IV placement and scan went very well and Jacob was very happy that he didn't wet his pants, lol (if you have never had a CT scan with contrast, the contrast makes you feel all warm and gives you the lovely sensation that you have wet your pants)! By 10:30 Jacob was retrieved by his MRI nurse, given juice and a snack and sent to a room to wait for the last part of his scan. Brie, Gavin and I were back in the lobby, waiting. Because Jacob fell asleep in this second half of his MRI, it went a lot quicker. Evidently, they can get better pictures and quicker when the patient is completely still. Duh. I always remind Jacob of this, but I guess he can't help but wiggling some. Jacob was done by 11:40 and the techs said he did a great job. We got lunch and headed to the hematology and oncology clinic. Our appt was at 11:30, but they don't mind if you are late due to another appointment. We ate and waited and everyone in our party seemed to be in good spirits given that we were on the last leg of our trip.

Even though they gave me the bad news first, I will start here with the good news. Jacob's MRI imaging of his brain, neck and brachial plexus showed no changes from the January 2011 scan. This is fantastic news! Either Jacob's tumors are on strike or the Avastin is doing it's job. The bilateral vestibular schwannomas have not grown and, since no growth is noted since the last scan, they are considered stable. Also, the right frontal lobe tumor has not changed. I am very thankful for this great report. They didn't get a great look at the ependymomas in the cervical spine because they were getting images of the brachial plexus, but they will scan them at NIH in August. Jacob will also have the brain and total spine scanned then and we will have more information.

As I mentioned on Jacob's Caring Bridge yesterday, the CT scan was to look at an adrenal gland mass (tumor) they hadn't look at in a while. I was looking through Jacob's medical records recently and noticed that it had been several years since it was imaged last. I found it on a spinal report from 2006 and another mention that it was unchanged in 2007. I believe the staff said the last CT of the adrenal gland and kidney area was in 2009, but I'm pretty sure it was in 2006. I can't find the CT record in my things, though. My “things” are growing uncontrollably at this point, so it could very well be the case. Anyway, at that time the tumor measured at 6 mm and Dr. Smith had Jacob do a 24 hour urine test to rule out Pheochromocytomas which is a type of tumor of the adrenal glands that can release high levels of epinephrine and norepinephrine into the body. Those results were negative. Jacob's doctors were not concerned about it and said it could be imaged again in a few years, that they would watch it on the MRIs. I honestly think with everything else going on with Jacob that this adrenal gland mass was forgotten about since it wasn't red-flagged on any of Jacob's spinal MRIs.

I was disappointed that Dr. Smith was not in clinic today to meet with us, but we did see another pediatric oncologist, Lamis Eldjerou, MD. She pulled up Jacob's CT scan and her and Hillary went over the images with me. The 6 mm adrenal gland tumor has grown to be 60 mm, 10 times it's size since it was last scanned and now it is a cause for concern. On the up side, it isn't invading Jacob's kidneys, stomach or liver. 6 centimeters is pretty big though and it looks like it could start pushing on organs any day now, in my professional opinion! Hillary called Dr. Smith and ordered blood work and urine labs. They will once again be checking for metanephrines to rule out Pheochromocytoma and also, as a precaution, will be looking for markers of Neuroblastoma. They do not believe it appears to be cancer, but they have to check. The tumor also has some calcification. I will have more information after the labs return. There are a few cases of adrenal gland tumors seen in Neurorfibromatosis type 1. Even though Jacob's genetics were negative for the NF1 mutation and positive for the NF2 mutation he still has several symptoms similar to NF1, like his plexiform brachial plexus tumor. Hilliary also contacted Dr. Asthagiri and he wants to see the scans. The scans will be sent to pediatric surgery for a consult to see if they recommend having it removed now. We also discussed adding a second chemotherapy drug to treat the adrenal gland tumor instead of surgery. If Jacob has surgery, he has to go off of the Avastin for several weeks before and after. TD did find online where if an adrenal gland tumor is under 10 cm it can usually be removed via laparoscopic surgery. We are only speculating though. Like I said, we will know more after the labs return and the other tumor board of doctors review Jacob's case. I hope this will be done in the next few days.

The cherry on top of our pile of bad news today is that on July 15, 2011, Dr. Amy Smith and the entire Pediatric Brian Tumor Program is moving from UF Shands in Gainesville, FL to Arnold Palmer Children's Hospital in Orlando, FL. This includes not only Jacob's oncologist of eight years, but also his beloved nurse practitioner, Hillary, and fantastic social worker, Michelle. I'm not sure what nurses will be leaving as well, but we love all of them. I am completely crushed. Years ago when no one knew what Jacob's diagnosis was or how to best treat him, Dr. Smith took charge and has held our hand through this difficult journey. Through tears I told Hillary that they are the glue to Jacob's puzzle and I will be lost with out them. I know that Dr. Smith and her team are best qualified to take on Jacob's complex case. I am thankful that we do have the option to stay with them, but I don't know how we will do this with lack of time and funds. Orlando is four hours from our home, one way, and with Jacob going for treatments every other week it would be a great burden. I will be putting a lot of thought and prayer into making the right decision over the next month. Hillary did tell us that Jacob could stay at Shands and they will have a new pediatric oncologist on staff, but not the brain tumor program and group. I do not want to start over, but we will have to consider it as an option. I will be doing more research over the next few weeks and we will do the best we can for Jacob.

After all of this news Jacob is, well, being Jacob. Other than his port being accessed without being numb today (twice), he has no complaints (he had the unexpected blood work this afternoon). He has named his adrenal gland tumor Franklin and he made the comment to me that he knows it could be worse. He said, "I'm ok. I'm not going to worry about it." Driving home this afternoon feeling completely distraught, I looked over at Jacob with his basketball shorts pulled up to his boxers line (sunning his legs), earphones in, singing way out of tune to some shot out country song and I just laughed out loud. I thanked God for this wonderful and unique young man who is teaching me so much (and it isn't “How to be Weird!”). He's just going with the flow, living and basking in the sun, so to speak, at every chance he gets. I love him so much.

Please continue to pray for him. Please pray for me and his doctors. For all of us to do what is best for Jacob.


  1. I just wanted you to know that Jacob and your family are always in our thought and prayers each day. My son has a heart condition and I know how frustrating those apt can be. We do not have as many to go as we used to but I for sure feel your pain. If you dont mind me asking what is Jacobs caring bridge page. I follow a coupld other kids that were in the PICU when my son was and it is so great to hear the story when they are doing well, and know that we need to pray extra hard when times are not so good.

  2. Still praying Tammy! Our God is amazing!